Objective: To describe the ocular ultrasound biomicroscopy (UBM) findings i
n patients with cystinosis.
Methods: Six patients with infantile nephropathic cystinosis, aged 16 to 25
years, and 6 controls (matched for age and spherical refractive error) wer
e examined clinically and with UBM. Scleral reflectivity, corneal and iris
thickness, central anterior chamber depth, angle width, trabecular meshwork
to ciliary process distance, and ciliary sulcus width were measured.
Results: No patient had glaucoma or posterior synechiae, but all had crysta
ls in the trabecular meshwork apparent with gonioscopy. Using UBM, the corn
ea and iris appeared similar in both groups, but the scleral reflectivity w
as increased in patients (P = .003). The angle was narrower in patients (me
an +/- SD, 20 degrees +/- 7 degrees) than controls (31 degrees +/- 5 degree
s, P < .001). The anterior chamber was shallower in patients (2556 +/- 197
mu m) than controls (2968+/-284 mu m, P < .001). The ciliary sulcus was clo
sed or narrow in all patients (83 +/- 112 mu m) compared with controls (339
+/- 135 mu m, P < .001), with a reduction in the trabecular meshwork to ci
liary process distance.
Conclusions: This report of ocular UBM findings in cystinosis demonstrated
narrowing of the angle and a ciliary body configuration similar to that rep
orted for plateau iris syndrome. Gonioscopy demonstrated crystals in the tr
abecular meshwork. These findings may explain the predisposition of these p
atients to glaucoma.